Department of Medicine, Section of Endocrinology and Metabolism, Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA.
World Neurosurg. 2013 Feb;79(2):404.e11-6. doi: 10.1016/j.wneu.2011.04.018. Epub 2011 Nov 10.
Pituitary carcinomas are rare and challenging clinical entities. Because of the paucity of cases, there is limited information in the literature on how best to diagnose and treat pituitary carcinomas.
We review the literature and describe a woman who presented with an adrenocorticotropic hormone (ACTH)-secreting pituitary macroadenoma that later evolved into a carcinoma with intracranial metastases.
A 39-year-old woman presented at age 27 with classic findings of Cushing's syndrome and a pituitary macroadenoma. Her initial treatment was transsphenoidal surgery, during which we confirmed an ACTH-secreting pituitary neoplasm. For 5 years, she was asymptomatic before her first recurrence. During the next 6 years, she underwent four transsphenoidal surgeries and two craniotomies. After each surgery, there was some reduction in the size of the macroadenoma but the residual tumor mass would rapidly enlarge. Immunochemical staining was positive for ACTH, and a stain for Ki-67 antigen showed a high mitotic index. Eleven years after her initial presentation, magnetic resonance imaging revealed bilateral hippocampal and tempero-occipital masses. The patient's health continued to deteriorate, largely from complications of severe hypercortisolemia, and she died from sepsis. At postmortem, the hippocampus and tempero-occipital lobe masses proved to be a pituitary tumor with positive ACTH staining.
Pituitary carcinomas are rare, may present many years after diagnosis of a primary pituitary adenoma, and should be suspected in patients with persistent or recurrent disease. Reliable histopathologic ways to distinguish between carcinoma and adenoma are difficult because the features of hypercellularity, nuclear pleomorphism, and mitotic figures are not always helpful.
垂体癌是罕见且具有挑战性的临床实体。由于病例稀少，文献中关于如何最好地诊断和治疗垂体癌的信息有限。
我们回顾了文献，并描述了一位患有促肾上腺皮质激素（ACTH）分泌性垂体大腺瘤的女性，后来发展为伴有颅内转移的癌。
一位 39 岁的女性在 27 岁时出现典型的库欣综合征表现和垂体大腺瘤。她最初的治疗是经蝶窦手术，在此期间我们证实了一种 ACTH 分泌性垂体肿瘤。5 年来，她无症状，直到第一次复发。在接下来的 6 年里，她接受了四次经蝶窦手术和两次开颅手术。每次手术后，大腺瘤的大小都有所缩小，但残留的肿瘤很快又会增大。免疫化学染色 ACTH 阳性，Ki-67 抗原染色显示高有丝分裂指数。在最初发病 11 年后，磁共振成像显示双侧海马和颞枕叶肿块。患者的健康状况持续恶化，主要是由于严重皮质醇增多症的并发症，最终死于败血症。尸检时，海马和颞枕叶肿块被证实为垂体肿瘤，ACTH 染色阳性。
垂体癌罕见，可能在原发性垂体腺瘤诊断后多年出现，应怀疑患有持续性或复发性疾病的患者。可靠的组织病理学方法很难区分癌和腺瘤，因为细胞增多、核异型性和有丝分裂象的特征并不总是有帮助。
